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Periodic fever aphtous stomatitis pharyngitis

Federica Vanoni, Silvia Federici, Jordi Antón, Karyl S Barron, Paul Brogan, Fabrizio De Benedetti, Fatma Dedeoglu, Erkan Demirkaya, Veronique Hentgen, Tilmann Kallinich, Ronald Laxer, Ricardo Russo, Natasa Toplak, Yosef Uziel, Alberto Martini, Nicolino Ruperto, Marco Gattorno, Michael Hofer
BACKGROUND: Diagnosis of Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is currently based on a set of criteria proposed in 1999 modified from Marshall's criteria. Nevertheless no validated evidence based set of classification criteria for PFAPA has been established so far. The aim of this study was to identify candidate classification criteria PFAPA syndrome using international consensus formation through a Delphi questionnaire survey. METHODS: A first open-ended questionnaire was sent to adult and pediatric clinicians/researchers, asking to identify the variables thought most likely to be helpful and relevant for the diagnosis of PFAPA...
April 18, 2018: Pediatric Rheumatology Online Journal
Perrine Dusser, Véronique Hentgen, Bénédicte Neven, Isabelle Koné-Paut
INTRODUCTION: PFAPA syndrome is the most frequent periodic fever syndrome in non-Mediterranean patients. The pathogenesis is unclear and the treatment is purely symptomatic and not standardized. The aim of this study was to assess colchicine's efficacy as prophylactic treatment in PFAPA syndrome and to identify factors able to predict response to treatment. METHODS: We performed a retrospective, multicentric, cohort study of PFAPA patients under colchicine prophylaxis...
July 2016: Joint, Bone, Spine: Revue du Rhumatisme
Ulla Lantto, Petri Koivunen, Terhi Tapiainen, Virpi Glumoff, Pasi Hirvikoski, Matti Uhari, Marjo Renko
Periodic Fever, Aphtous stomatitis, Pharyngitis, and Adenitis (PFAPA) is a childhood febrile syndrome that is often cured by tonsillectomy (TE). We hypothesized that microbes present in the tonsils may act as a trigger for the activation of inflammasomes and investigated the microbiology of the tonsils in PFAPA patients and controls. We recruited 31 consecutive children who underwent TE due to PFAPA; 24 children who underwent TE due to other reasons served as controls. We cultured all the samples for bacteria, mycobacteria, yeasts, and viruses and used PCR for 15 viruses...
June 2015: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Sandra Berody, Caroline Galeotti, Isabelle Koné-Paut, Maryam Piram
UNLABELLED: Mevalonate kinase deficiency (MKD) is an autosomic recessive auto-inflammatory disease caused by mutations of the MVK gene. MKD being a very rare disease, numerous misdiagnoses and medical referrals may precede the right diagnosis, amplifying the burden of the disease. OBJECTIVES: To evaluate the patient's medical referrals between the first symptom and the diagnosis of MKD and the diagnosis delay. METHODS: A questionnaire was sent to French paediatric and adult rheumatologists to retrospectively collect information from genetically confirmed patients with MKD regarding the first symptoms of the disease, the different diagnoses made previously, the treatments received, and the disease burden evaluated mainly by the number of hospitalizations...
July 2015: Joint, Bone, Spine: Revue du Rhumatisme
Petra Dytrych, Petra Krol, Dytrych Petra, Krol Petra, Michaela Kotrova, Daniela Kuzilkova, Kotrova Michaela, Kuzilkova Daniela, Petr Hubacek, Ladislav Krol, Hubacek Petr, Krol Ladislav, Rami Katra, Ondrej Hrusak, Katra Rami, Hrusak Ondrej, Zdenek Kabelka, Pavla Dolezalova, Kabelka Zdenek, Dolezalova Pavla, Tomas Kalina, Eva Fronkova, Kalina Tomas, Fronkova Eva
PURPOSE: PFAPA syndrome is a benign, recurrent inflammatory disease of childhood. Tonsillectomy is one of the therapeutic options with a yet unexplained biological mechanism. We tested whether specific lymphocyte subsets recruited from blood to human tonsils participate in PFAPA pathogenesis. METHODS: Paired tonsils/peripheral blood (PB) samples were investigated (a) from children with PFAPA that successfully resolved after tonsillectomy (n=10) (b) from children with obstructive sleep apnoea syndrome as controls (n=10)...
May 2015: Molecular Immunology
Petra Król, Marek Böhm, Viktor Sula, Petra Dytrych, Rami Katra, Dana Nemcová, Pavla Dolezalová
OBJECTIVES: This paper aims to describe clinical and laboratory features and disease outcome in a single-centre cohort of patients with PFAPA syndrome (Periodic Fever, Aphtous stomatitis, Pharyngitis, and Adenitis) and to test performance of diagnostic and therapeutic algorithms. METHODS: Patients fulfilling criteria were selected from the fever clinic population. Prospective follow-up together with recruitment of newly diagnosed patients followed pre-defined guidelines...
November 2013: Clinical and Experimental Rheumatology
Marco Colotto, Marianna Maranghi, Cosimo Durante, Marco Rossetti, Alessandra Renzi, Maria Grazia Anatra
Since its clinical definition in 1987, the syndrome called, "periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis" syndrome (PFAPA) has been considered peculiar to pediatric age. In the recent literature there are a few case reports of PFAPA in adults. We describe a case of a 21-year-old female affected by PFAPA who presented a history of tonsillectomy at the age of four. To our knowledge this is the fourth case described with a diagnosis of PFAPA in an adult with a history of tonsillectomy during childhood...
2011: Internal Medicine
R Ciambra, C Milocco, S Facchini, V Leone, C Locatelli, M Pocecco
We report 5 patients (3F; 2M; age 19-60 months) affected by a syndrome characterized by recurrent episodes of abrupt onset of fever, aphtous stomatitis, pharyngitis and cervical adenopathy named by the acronym PFAPA (periodic fever, aphtous stomatitis, pharyngitis and adenopathy). The episodes had clockwork periodicity (3-4 weeks) and the fever was unaccompanied by remarkable respiratory signs or symptoms. All children were doing well between episodes. The inflammatory markers were elevated only during attacks in all children...
May 2003: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
S Hernández-Bou, M Giner, A M Plaza, J I Sierra, M A Martín Mateos
BACKGROUND: PFAPA syndrome (Periodic Fever, Aphtas, Pharyngitis and cervical Adenopathies) is one of the causes of periodic fever in pediatrics and it is characterised by high fever, pharyngitis, cervical adenitis and aphtous stomatitis. Its etiopathogeny is unknown. The diagnosis is clinical and the findings of laboratory are unspecified. One or two doses (1 mg/kg) of oral prednisone are enough for a fast resolution of the clinic. It is a benign syndrome and no sequels have been noticed after its disappearance, usually in four years from its beginning...
July 2003: Allergologia et Immunopathologia
P Ovetchkine, M L Bry, P Reinert
Marshall's syndrome or periodic fever syndrome was first described in 1987 in the USA based on observations of 12 children under the age of five with periodic fever (> 38 degrees C) and accompanying aphtous stomatitis, pharyngitis, and cervical adenopathy (PFAPA). In 1998, a national retrospective study was carried out in France by the pediatric infectious pathology group, and a semeiological analysis was made of 22 cases. The main characteristics of Marshall's syndrome found in this patient population were in agreement with those reported in the literature...
June 2000: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
J P Drenth, N E Denecker, A M Prieur, J W Van der Meer
The hyper-IgD syndrome is a rare entity characterized by early onset of attacks of periodic fever. All patients have an elevated serum IgD (> 100 U/ml). Symptoms during attacks include joint involvements (arthralgias/arthritis), abdominal complaints (vomiting, pain, diarrhoea), skin lesions, swollen lymph nodes, and headache. In 1992 an International hyper-IgD study group was established, and to date the diagnosis has been made in 60, mainly European patients; 14 come from France. The disorder occurs in families and is transmitted by autosomal recessive inheritance...
September 16, 1995: La Presse Médicale
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